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Reliability of Echocardiographic pulmonary vascular resistance to Screen for the New Definition of Precapillary pulmonary Hypertension in Uncorrected Secundum Atrial Septal Defect

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Congenital Heart Disease 2024年第3期19卷 315-324页

作者： Risalina Myrtha Hasanah Mumpuni Real Kusumanjaya Marsam Dyah Wulan Anggrahini Anggoro Budi Hartopo Lucia Kris Dinarti Department of Cardiology and vascular Medicine Faculty of MedicineUniversitas Sebelas MaretSurakarta57126Indonesia Department of Cardiology and vascular Medicine Universitas Sebelas Maret HospitalSukoharjo57161Indonesia Department of Cardiology and vascular Medicine Faculty of MedicinePublic Healthand NursingUniversitas Gadjah MadaYogyakarta55281Indonesia Department of Cardiology and vascular Medicine Dr.Sardjito HospitalYogyakarta55284Indonesia

Background and Objective:The most feared complication of uncorrected secundum Atrial Septal Defect(ASD)is pulmonary arterial hypertension(PAH).pulmonary vascular resistance(PVR)is crucial in detecting precapil-lary pulmonary hypertension(PH)to guide the need for PAH-specific *** is a change in the cut-off value of PVR according to the recently updated PH *** echocardiographic PVR(PVRecho)correlates to PVR by right heart catheterization(RHC)(PVRcath)according to the new guidelines has not been *** aim of this study is to determine the reliability of PVRecho in detecting PAH in Uncorrected Ostium Secundum ASD based on the current updated guideline and to help screen the high PVR ***:429 ostium secun-dum ASD in the COngenital HeARt Disease in Adult and pulmonary Hypertension(COHARD-PH)registry was divided into three groups according to the *** was calculated using Abbas’Formula and compared the its gold standard,the *** correlation between the two methods was *** Bland-Altman plot was used to analyze the agreement between the two *** operating characteristics(ROC)analysis was used to determine the PVRecho cut-off value for high ***:The majority of the population(63.5%)had high *** gender dominated the study population(84%).PVR_(echo) was significantly correlated with PVRcath(r=0.6225,p1.62 WU showed good dis-criminatory power in determining high PVR.

关键词： pulmonary vascular resistance atrial septal defect echocardiography

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Development of pulmonary hypertension remains a major hurdle to corrective surgery in Down syndrome

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World Journal of Cardiology 2024年第1期16卷 1-4页

作者： Akash Batta Juniali Hatwal Department of Cardiology Dayanand Medical College and HospitalLudhiana 141001PunjabIndia Department of Internal Medicine Post Graduate Institute of Medical Education&ResearchChandigarh 160012India

Down syndrome is the most common chromosomal abnormality encountered in clinical practice with 50%of them having associated congenital heart disease(CHD).Shunt lesions account for around 75%of all CHDs in Down *** syndrome patients,especially with large shunts are particularly predisposed to early development of severe pulmonary hypertension(PH)compared with shunt lesions in general *** necessitates timely surgical correction which remains the only viable option to prevent long term morbidity and ***,despite clear recommendations,there is wide gap between actual practice and fear of underlying PH which often leads to surgical refusals in Down syndrome even when the shunt is *** peculiarity is that Down syndrome patients can develop PH even after successful correction of *** is not uncommon to come across Down syndrome patients with uncorrected shunts in adulthood with irreversible PH at which stage intracardiac repair is contraindicated and the only option available is a combined heartlung ***,despite the guidelines laid by authorities,the rates of cardiac transplant in adult Down syndrome remain dismal largely attributable to the high prevalence of intellectual disability in *** index case presents a real-world scenario highlighting the impact of severe PH on treatment strategies and discrimination driven by the fear of worse outcomes in these patients.

关键词： Down syndrome Congenital heart disease pulmonary hypertension Cardiac transplantation pulmonary vascular resistance Surgical correction

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Efficacy of Bosentan in Patients after Fontan Procedures：a Double-blind, Randomized Controlled Trial

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Journal of Huazhong University of Science and Technology(Medical Sciences) 2016年第4期36卷 534-540页

作者：尚小珂卢蓉张喜张长东肖书娜柳梅王斌董念国 Cardiac Surgery Union Hospital Tongji Medical College Huazhong University of Science and Technology Wuhan 430022 China SinoScript LLC Vernon Connecticut 06066 USA Department of Intervention Wuhan Asia Heart Hospital Wuhan 430022 China Department of Radiology The Keck School of Medicine University of Southern California California 90033 USA

Fontan surgery is a widely used palliative procedure that significantly improves the survival period of patients with complex congenital heart disease（CHD）. However, it does not decrease postoperative complication rate. Previous studies suggested that elevated mean pulmonary artery pressure（m PAP） and vascular resistance lead to decreased exercise tolerance and myocardial dysfunction. Therapy with endothelial receptor antagonists（Bosentan） has been demonstrated to improve the patients＇ prognosis. A double-blind, randomized controlled trial was performed to explore the efficacy of Bosentan in treating patients who underwent the Fontan procedure. Eligible participants were randomly divided into Bosentan group and control group. Liver function was tested at a local hospital and the results were reported to the phone inspector every month. If the results suggested abnormal liver function, treatment would be adjusted or terminated. All the participants finished the follow-up study, with no patients lost to follow-up. Unblinding after 2-year follow-up, no mortality was observed in either group. However, secondary end-points were found to be significantly different in the comparable groups. The cardiac function and 6-min walking distance in the Bosentan group were significantly superior to those in the control group（P=0.018 and P=0.027）. Bosentan could improve New York Heart Association（NYHA） functional status and improve the results of the 6-min walking test（6MWT） in Fontan patients post-surgery, and no other benefits were observed. Furthermore, a primary meta-analysis study systematically reviewed all the similar clinical trails worldwide and concluded an overall NYHA class improvement in Fontan patients who received Bosentan treatments.

关键词： Fontan procedure Bosentan prognosis pulmonary hypertension pulmonary vascular resistance

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Echocardiogram in predicting correctable shunts in ventricular septal defect patients associated with severe pulmonary hypertension

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South China Journal of Cardiology 2023年第4期24卷 175-181页

作者： LI He-zhi HUANG Ze-han FEI Hong-wen ZHANG Cao-jin Department of Cardiology Guangdong Cardiovascular InstituteGuangdong Provincial People's HospitalGuangdong Academy of Medical SciencesGuangzhou510030China

Background Echocardiogram is a simple and useful tool in disease *** ventricular septal defect(VSD)patients with severe pulmonary hypertension(PH),it is difficult to judge whether further intervention is ***,this study aimed to investigate the application of echocardiogram in predicting the severity of pulmonary hypertension and guide subsequent *** This study included VSD patients who underwent right heart catheterization(RHC)examination in Guangdong Provincial People's Hospital from January2011 to December *** estimated pulmonary artery systolic pressure(e PASP)higher than 60 mm Hg was defined as severe PH in this *** regression analysis and receiver operating characteristic curve(ROC)analysis were *** A total of 186 VSD patients with severe PH(e PASP more than 60 mm Hg)were included in this study,with 158 cases in the non-correctable group and 28 cases in the collectable *** univariable logistic regression,left atrium dimension(LA),left ventricular end-diastolic dimension(LVDd),left ventricular end-systolic dimension(LVDs),peak velocity of the pulmonary valve(PV),peak velocities from the early phase of the mitral inflow(MVE),bidirectional shunting and pericardial effusion were associated with a correctable *** adjusted in multivariable model,only PV and bidirectional shunting remained statistically *** ROC curve found that PV exhibits good discriminative ability for correctable shunt(AUC[area under the curve]:0.779,95%CI:0.676-0.871)with a cut-off value of 1.465 m/*** predictive performance of bidirectional shunting was not satisfactory,with a low AUC of 0.669(95%CI:0.571-0.766).Conclusions PV and bidirectional shunting are simple and clinically available parameters from echocardiogram in predicting PH severity,which not only avoids the repeated unnecessary cardiac catheterization,but also provides a reference basis for follow-up evaluation.

关键词： Ventricular septal defect pulmonary hypertension pulmonary vascular resistance Echocardiogram Congenital heart disease

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